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Objective@#To explore the relationship between the cognitive impairment and cerebral lesions using 7.0 Tesla magnetic resonance imaging (MRI) in CADASIL patients. @*Methods@#Thirty five CADASIL patients confirmed by serum NOTCH3 gene detection in Peking University First Hospital from June 2015 to November 2018 were enrolled, including 19 males and 16 females, of which the age of onset was (39.28±8.31) years, the age of admission was (44.61±8.42) years, and the course of disease was (5.29±3.65) years. 7.0 Tesla MRI was performed in all the patients. The numbers of lacunar infarcts and microbleeds were counted and the white matter changes were evaluated with age-related white matter rating scale (ARWMrs). Neuropsychological tests were used to evaluate the global cognition, memory, attention, executive function, visuo-spatial function and language function separately. The z score was calculated to evaluate the impairment extent in different scales. The correlation analysis was performed between image changes and neuropsychological tests. Thirty nine normal controls including 20 males and 19 females with age of (42.54±8.92) years were also enrolled, and the same neuropsychological tests were performed in these subjects. @*Results@#The numbers of microbleeds and lacunar infarcts were 13.71±10.29 and 5.89 (8.74). The ARWMrs score was 11.26±5.31. There were 21 patients (60%) presented with cognitive impairment. In comparison with the controls, the patients presented with global cognitive impairment (MMSE score 26.87±3.95 vs 29.08±0.95), including executive (finishing time of Stroops-c: 80.00 (103.75) s vs 67.79 (16.00) s, correct number of Stroops-c: 48.00 (44.26) vs 50.00 (2.00), time of trail making A test: 55.5 (81.5) s vs 39.0 (5.0) s, time of trail making B test: 171.0 (159.5) s vs 103.0 (54.0) s, false number of trail making B test: 0(2) vs 0(0)), memory (number of register memory: 16.13±5.41 vs 21.1±15.21, number of long term recall: 4.78±2.83 vs 7.41±2.24, number of cue recall memory: 4(6) vs 8(4), number of recognition memory: 10.00 (2.25) vs 11.00 (2.00)), attention (number of digital span: 4.42±1.46 vs 7.89±1.65, correct number of symbol digitalis modality test: 38.47±17.29 vs 51.41±13.00), visuo-spatial (Rey-osterrich: 34 (5) vs 36 (2)) and language function (number of semantic fluency: 14.70±5.54 vs 17.46±5.63) (P<0.05). The z score demonstrated impaired executive function, followed by visuo-spatial dysfunction. The number of lacunar infarcts and microbleeds significantly correlated with short term recall memory (r=-0.404, -0.393), long term recall memory (r=-0.375, -0.395), cue memory (r=-0.395, -0.437), Stroops-c time (r=0.412, 0.503), trails making A test time (r=0.400, 0.434)(P<0.05). The number of lacunar infarcts significantly correlated with symbol digitalis modality test (r=-0.475) (P<0.05). The number of microbleeds significantly correlated with digital span test (r=-0.390), Boston naming test (r=-0.382) and semantic fluency (r=-0.449) (P<0.05). ARWMrs score significantly correlated with MMSE score (r=-0.357), rigister memory (r=-0.342), trails making A finishing time (r=0.425), trails making B finishing time (r=0.463) and correct numbers of trails making B (r=0.392) (P<0.05). @*Conclusions@#CADASIL presented with global cognitive impairment, especially executive function and visuo-spatial function. The white matter changes, lacunar infarcts and microbleeds affected different cognitive function.
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Objective To investigate the effect of cardiac function and renal function on early neurological function recovery of acute stroke patients.Methods Hospitalized acute stroke patients from January 2010 to May 2018 were recruited in the Department of Neurology,Peking University First Hospital,who were divided into two groups according to the modified Rankin Scale (mRS) score at discharge,good recovery (mRS score ≤2) and poor recovery (mRS score ＞2).The clinical characteristics,laboratory and echocardiography data were collected respectively.All cases were classified according to Trial of Org 10 172 in Acute Stroke Treatment (TOAST) criteria.Estimated glomerular filtration rate (eGFR) was calculated by chronic kidney disease epidemiology collaboration equation in renal function assessment,and cardiac function was calculated by left ventricular ejection fraction (LVFF) and E-wave/A-wave ratio (E/A).Multivariate Logistic regression analysis and stratified analysis were performed to explore the effects of cardiac and renal function and interactions on stroke patients.Results A total of 517 patients with acute stroke were enrolled in this study,23.4％ (121/517) of which presented with chronic kidney disease.Poor recovery was aggravated with the progression of eGFR stage in patients with acute stroke (x2=14.627,P=0.001).Compared with the good recovery group,LVEF and E/A were significantly lower in the poor recovery group,while left atrium diameter ((3.87±0.52) cm vs (3.77±0.49) cm,t=-2.139,P=0.033),interventricular septum thickness ((1.10±0.19) cm vs (1.04±0.16) cm,t=-4.056,P=0.000),left ventricular posterior wall thickness ((1.00±0.13) cm vs (0.98±0.13) cm,t=-2.190,P=0.029) and left ventricular mass index ((102.03±25.73) g/m2 vs (94.94±23.63) g/m2,t=-3.145,P=0.002) were significantly higher in the poor recovery group.Stratified analysis showed that the rate of poor recovery increased with the decrease of eGFR at different levels of LVEF and E/A.Compared with patients of normal renal function and mild impairment of renal function or with patients of high third tertile of LVEF,chronic kidney disease significantly increased the rate of poor recovery in patients with low third tertile of LVEF,while the rate of poor recovery was not significantly different in patients with chronic kidney disease among third tertile of E/A.The levels of eGFR and LVEF in different stroke subtypes were significantly different (F=7.433,P=0.000;F=2.617,P=0.034).The eGFR and LVEF levels of the cardioembolism (CE) group were the lowest compared with other subtypes of stroke.The eGFR levels in the CE group were significantly lower than that in other subtypes except the large artery atherosclerosis group,and the LVEF level was significantly lower in the CE group compared to the small artery occlusion group.Multivariate Logistic regression analysis revealed that eGFR (OR=0.866,95％CI 0.760-0.987,P=0.031),LVEF (OR=0.798,95％CI 0.688-0.925,P=0.003),E/A (OR=0.136,95％CI 0.034-0.548,P=0.005) and eGFR by LVEF (OR=1.002,95％CI 1.000-1.004,P=0.022) were significantly associated with early functional outcome in patients with acute stroke after adjusting for relevant clinical confounders (all P＜0.05).Conclusions Renal function,cardiac systolic and diastolic function were associated with the early functional recovery in patients with acute stroke.Moreover,cardiac systolic dysfunction and renal dysfunction interacted significantly with the early neurological function recovery in patients with acute stroke.
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The patient is a young woman,manifested as orthostatic hypotension and gastrointestinal motility disorders in acute onset.The physical examination and laboratory test suggested disorders of wide range of autonomic neuropathy.The levels of serum antinuclear antibody and SSA antibody were elevated.The biopsy of lip gland suggested Sjogren's syndrome.Nerve biopsy showed loss of a large number of unmyelinated nerve fibers.After the treatment of intravenous gamma globulin and glucocorticoid and symptomatic treatment,the symptoms of orthostatic hypotension were significantly relieved,but the gastrointestinal motility was not significantly improved.
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Objective To characterize the clinical manifestations, laboratory findings of patients with occult sys?temic malignant neoplasms, whose initial manifestation presented as multiple acute cerebral infarcts including coagula?tion function,radiological imaging and microembolic signals (MES) detection by transcranial Doppler sonography (TCD) and to explore the possible underlying mechanisms. Methods All clinical records, laboratory hematological tests includ?ing hypercoagulable states measured by D-dimer levels, brain MRI including DWI, and TCD monitoring MES, the treat?ment and prognosis were retrospectively reviewed in 12 patients with multiple acute cerebral infarcts as the first manifes?tation of occult systemic malignancy. Results The clinical manifestations presented as localized neurological dysfunction, e.g. hemiparesis, aphasia, hemiparesthesia, dysarthria, vertigo and seizures, etc. DWI revealed multiple disseminated acute cerebral infarcts in multiple arterial territories such as the bilateral anterior or anterior plus posterior cerebral circu?lation in all patients. Eleven of 12 patients tested had elevated D-dimer. TCD detected MES in 5 of 7 patients. There were 12 patients diagnosed with occult systemic malignancy including 5 lung cancer, 3 pancreatic cancer, 1 gastric can?cer, 1 colon cancer, 1 endometrial adenocarcinoma and 1 metastatic poorly differentiated mucinous adenocarcinoma with unknown primary. Ten patients already had remote metastasis at diagnosis. The prognosis was usually poor and there were 7 cases with ischemic stroke recurrence, 4 cases with acute myocardial infarction, 3 cases died during hospitaliza?tion. Conclusions When patients present with multiple disseminated acute cerebral infarcts involving multiple arterial territories as initial manifestation, the underlying occult systemic malignancy should be considered. Hypercoagulopathy and MES might provide the clues to the diagnosis.
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Objective To investigate the clinical features and TCD-detected microembolic signals in patients with hypercoagulability related multiple acute cerebral infarcts within non-single arterial territories, and to explore the possi?ble underlying mechanisms. Methods A retrospective review was conducted on all clinical, laboratory, radiological and TCD monitoring records from patients with hypercoagulability related multiple acute cerebral infarcts within non-single arterial territories, who admitted to the neurology department in our hospital. Results The data from twenty-two cases were finally included in this study. All patients presented with acute-onset localized neurological dysfunction, e.g. hemi?paresis, aphasia, hemiparesthesia, dysarthria, hemianopsia and cortical blindness. Their hypercoagulability related diseas?es included 10 cases of systemic malignancy, 5 moderate to severe hyperhomocystynemia (HCY>50μmol/L), 2 nephrot?ic syndrome, 2 antiphospholipid syndrome, 1 ulcerative colitis, 1 polycythemia vera,1 paroxysmal nocturnal hemoglobin?uria. In 18 cases, the hypercoagulability related diseases were diagnosed after their initial stroke onset. DWI showed mul?tiple disseminated acute cerebral infarcts in non-single arterial territories involving bilateral anterior or anterior plus pos?terior cerebral circulation simultaneously. Foci involved lobar cortex/subcortex of cerebral hemisphere in 22 cases, deep cerebral hemisphere in 12 cases, cerebellum foci in 10 cases,brainstem foci in 2 cases. TCD revealed microembolic sig? nals in ten of 22 patients monitored. Conclusions Patients with multiple acute cerebral infarcts involving non-single arte?rial territories, should be screened for hypercoagulability as in that hypercoagulability and microembolism might be in?volved in the etiology of cerebral infarction.